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    MTO1 tRNA modification protein MTO1 [ Saccharomyces cerevisiae S288C ]

    Gene ID: 852616, updated on 13-Apr-2024

    Summary

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    Gene symbol
    MTO1
    Gene description
    tRNA modification protein MTO1
    Primary source
    SGD:S000003205
    Locus tag
    YGL236C
    See related
    AllianceGenome:SGD:S000003205
    Gene type
    protein coding
    RefSeq status
    REVIEWED
    Organism
    Saccharomyces cerevisiae S288C (strain: S288C)
    Lineage
    Eukaryota; Fungi; Dikarya; Ascomycota; Saccharomycotina; Saccharomycetes; Saccharomycetales; Saccharomycetaceae; Saccharomyces
    Also known as
    IPS1
    Summary
    Predicted to enable flavin adenine dinucleotide binding activity. Involved in mitochondrial tRNA wobble uridine modification. Located in mitochondrion. Used to study nonsyndromic deafness. Human ortholog(s) of this gene implicated in combined oxidative phosphorylation deficiency 10. Orthologous to human MTO1 (mitochondrial tRNA translation optimization 1). [provided by Alliance of Genome Resources, Apr 2022]
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    Genomic context

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    Location:
    chromosome: VII
    Exon count:
    1
    Sequence:
    Chromosome: VII; NC_001139.9 (53787..55796, complement)

    Chromosome VII - NC_001139.9Genomic Context describing neighboring genes Neighboring gene importin-alpha export receptor Neighboring gene transcription activator HAP2 Neighboring gene uncharacterized protein Neighboring gene bifunctional aminoimidazole ribotide synthase/glycinamide ribotide synthase Neighboring gene Rab GTPase-binding exocyst subunit SEC15

    Genomic regions, transcripts, and products

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    Genomic Sequence:
    NC_001139.9

    Go to nucleotide: Graphics FASTA GenBank

    Bibliography

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    Related articles in PubMed

    1. MTO1 worked as a modifier in the aminoglycosides sensitivity of yeast carrying a mitochondrial 15S rRNA C1477G mutation. Zhu X, et al. PLoS One, 2015. PMID 25898254, Free PMC Article
    2. Mutations of the mitochondrial-tRNA modifier MTO1 cause hypertrophic cardiomyopathy and lactic acidosis. Ghezzi D, et al. Am J Hum Genet, 2012 Jun 8. PMID 22608499, Free PMC Article
    3. Mutation in MTO1 involved in tRNA modification impairs mitochondrial RNA metabolism in the yeast Saccharomyces cerevisiae. Wang X, et al. Mitochondrion, 2009 Jun. PMID 19460296, Free PMC Article
    4. MTO1 codes for a mitochondrial protein required for respiration in paromomycin-resistant mutants of Saccharomyces cerevisiae. Colby G, et al. J Biol Chem, 1998 Oct 23. PMID 9774408
    5. Identification and characterization of mouse MTO1 gene related to mitochondrial tRNA modification. Li R, et al. Biochim Biophys Acta, 2003 Oct 1. PMID 14522080

    See all (38) citations in PubMed

    GeneRIFs: Gene References Into Functions

    What's a GeneRIF?
    1. The deletion of the nuclear gene MTO1 regulates neomycin sensitivity in yeast carrying the mitochondrial 15S rRNA C1477G mutation.
      Title: MTO1 worked as a modifier in the aminoglycosides sensitivity of yeast carrying a mitochondrial 15S rRNA C1477G mutation.
    2. The respiratory yeast phenotype for MTO1 was dramatically worsened in stress conditions and in the presence of a paromomycin-resistant (P(R)) mitochondrial rRNA mutation
      Title: Mutations of the mitochondrial-tRNA modifier MTO1 cause hypertrophic cardiomyopathy and lactic acidosis.
    3. These data strongly indicate that unmodified tRNA caused by the deletion of MTO1 gene caused the instability of mitochondrial tRNAs and mRNAs and an impairment of aminoacylation of mitochondrial tRNAs.
      Title: Mutation in MTO1 involved in tRNA modification impairs mitochondrial RNA metabolism in the yeast Saccharomyces cerevisiae.
    Submit: New GeneRIF Correction

    Pathways from PubChem

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    Interactions

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    Products Interactant Other Gene Complex Source Pubs Description

    General gene information

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    Homology

    Gene Ontology Provided by SGD

    Function Evidence Code Pubs
    enables flavin adenine dinucleotide binding IBA
    Inferred from Biological aspect of Ancestor
    more info
    		  
    enables flavin adenine dinucleotide binding IEA
    Inferred from Electronic Annotation
    more info
    		  
    enables molecular_function ND
    No biological Data available
    more info
    		  
    Process Evidence Code Pubs
    involved_in mitochondrial tRNA wobble uridine modification IBA
    Inferred from Biological aspect of Ancestor
    more info
    		  
    involved_in mitochondrial tRNA wobble uridine modification IMP
    Inferred from Mutant Phenotype
    more info
    		 PubMed 
    involved_in tRNA methylation IBA
    Inferred from Biological aspect of Ancestor
    more info
    		  
    involved_in tRNA processing IEA
    Inferred from Electronic Annotation
    more info
    		  
    involved_in tRNA wobble uridine modification IEA
    Inferred from Electronic Annotation
    more info
    		  
    Component Evidence Code Pubs
    is_active_in cytosol IBA
    Inferred from Biological aspect of Ancestor
    more info
    		  
    located_in mitochondrion HDA PubMed 
    is_active_in mitochondrion IBA
    Inferred from Biological aspect of Ancestor
    more info
    		  
    located_in mitochondrion IDA
    Inferred from Direct Assay
    more info
    		 PubMed 
    located_in mitochondrion IEA
    Inferred from Electronic Annotation
    more info
    		  

    General protein information

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    Preferred Names
    tRNA modification protein MTO1
    NP_011278.2
    • Mitochondrial protein; forms heterodimer complex with Mss1p that performs 5-carboxymethylaminomethyl modification of the wobble uridine base in mitochondrial tRNAs; required for respiration in paromomycin-resistant 15S rRNA mutants; human homolog MTO1 can complement yeast null mutant

    NCBI Reference Sequences (RefSeq)

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    Genome Annotation

    The following sections contain reference sequences that belong to a specific genome build. Explain

    Reference assembly

    Genomic

    1. NC_001139.9 Reference assembly

      Range
      53787..55796 complement
      Download
      GenBank, FASTA, Sequence Viewer (Graphics)

    mRNA and Protein(s)

    1. NM_001181102.1 → NP_011278.2  TPA: tRNA modification protein MTO1 [Saccharomyces cerevisiae S288C]

      See identical proteins and their annotated locations for NP_011278.2

      Status: REVIEWED

      UniProtKB/Swiss-Prot
      D6VV98, P53070
      UniProtKB/TrEMBL
      A0A8H4BZW5, A6ZTV4, B3LHS9, B5VIA7, C7GNY3, C8Z819
      Conserved Domains (1) summary
      TIGR00136
      Location:37 → 665
      gidA; glucose-inhibited division protein A
    Nucleotide Protein
    Heading Accession and Version
    Protein Accession Links
    GenPept Link UniProtKB Link
    P53070.2

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